Category: cystic fibrosis
Today is a day of firsts for Julia. It was her first day of three-year-old preschool and her first trip to the hospital for a clean-out. Let’s start with preschool…
Julia goes on safari on her first day of preschool!
Oakwood here I come!
Another first…Julia’s first clean-out. Julia is so brave. She has been handling things like a pro! Gotta love this girl…
Heading into PCMC for Julia's first clean-out
Julia waits for IV to be placed. She was so brave. She only cried after it was done...and she did call it, "Stupid!" The nurses could only agree.
Waiting for the PICC to be placed. Julia was starving...asking over and over for chicken strips and chocolate milk.
Julia now has a PICC, IV is now out and this girl got to eat some homemade chicken nuggets and chocolate milk!
I think the hardest times for me were when Julia got the IV placed and when I had to leave the room for the team to place her PICC. I teared up with her when she cried after her IV. She was so brave, but I could tell how scared she really was. The funny part was when Julia got her pre-PICC drugs. She had been complaining for over an hour about how hungry she was and how she wanted to go home, but the second the drugs hit her little body, she started laughing, just cracking up. I couldn’t help but laugh right along with her. I have video of a little bit of her laughing and her “happy” time. Ahhh, what great drugs they have here. Makes getting a PICC quite the happy, laughing experience. She shouldn’t remember any of the PICC line going in as they also gave her a “forgetting” drug along with the happy, relaxing drugs.
Please visit if you can. Julia will surely be very happy to see any familiar faces. We are in 3047 and can have visitors ’til 10 PM, but come before 9 as we will need to sleep. We start the treatment schedule tomorrow and it should include four treatments a day along with her intravenous antibiotics.
We truly feel of your love and support at this time as it has picked us up and carried us along. We couldn’t survive this without such loving, generous support. It brings tears to my eyes thinking about all of our family and friends who are truly making this difficult time just a bit more pleasant. We love you all and are so blessed to have you in our lives!
Wow, the past couple of weeks have been quite the adjustment! School, school, school…is all that is going on around here. School started for me last night. I am taking two classes, equaling six credits. One is a class on assessment of the reading process and the other is an early literacy instruction course. I am so excited about the material we’ll be covering in these classes as well as the opportunity to actually get back out into the field with some students as I fulfill my assignments. I will be working in an elementary school in Rose Park, with a demographic that I am most familiar with. It should feel like going back to Maple School and that’s what I’m so excited about. This semester will be filled with tons of hard work, but I look forward to experiencing some growth along the way. What a treat it is to finally be able to participate in some good adult, academic conversation! Even more exciting is that most of my classmates are kindergarten teachers! Yea for kindergarten! Way to represent!
I've officially traded the diaper bag for a backpack...
When Mom's away at school, Olivia keeps Julia company during treatment.
In other news, I had Julia’s preschool parent meeting today. I’d be lying if I said I wasn’t completely nervous about Julia going to preschool next week. I brought the two teachers the booklet provided to us by Primary Children’s Medical Center about CF and school. I told them to read it and to give me a call so we can set up a time to meet together to discuss Julia and answer questions. This is new and uncharted territory for me. We may need to have an IEP (Individualized Education Plan) drawn up just to cover everything in writing, but for now, I just want to meet with her teachers and go from there.
If any of you more experienced CF parents want to give me some advice, let me have it please. I’m open to anything and everything. I know how to send a kid to preschool, but not how to send a CF kid to preschool, not at all.
Julia is in for a big shocker when I walk out of the door and leave her at preschool. I’ve told her all about it, but I fully expect some drama. She has been enjoying having the house to herself when the older two are at school. The first day we dropped Olivia and Austin off at school, I said, “Well Julia, it’s just me and you.” Julia kept saying the rest of the morning, “It’s just me and you mom. What are we going to do?” She cracks me up!
Julia and I at playgroup...just me and Julia!
We are still waiting for Julia’s culture results, but I’m expecting the call any moment. The wait is killing me! I just want to know…
Julia leaving clinic at PCMC...nothing like a piece of your heart walking around outside your body.
Today was Julia’s CF clinic appointment. Every three months Julia has an appointment up at Primary Children’s Medical Center. These appointments help us give Julia the best care we possibly can. We had a great visit today! Julia has gained over three pounds in the last few months and has grown a few inches as well. She is tall and lean and completely average. This is great news! It’s easier for a CFer to stay healthy with a little meat on their bones.
Today was different because Julia is now old enough to stand on the big kid scale and be measured on the wall with the big kid ruler. She really hated the baby scale and the thing she had to lay down on to get her height.
We are also going to start practicing using a mouth piece for a few minutes during treatment time. A mouth piece is much more effective than a mask in delivering the inhaled medicines to her lungs. So, we will start using a nose plugger and working her slowly into using a mouth piece.
The disappointing news is that a clean-out is lingering in Julia’s very near future. We will be awaiting culture test results this week and will need to make some decisions based on what grows in her culture. For those who don’t know what a clean-out is…it’s a hospital stay that includes a PICC line placement and the use of very strong intravenous antibiotics. A PICC line administers the medicine right to the heart, so it’s pumped through the blood stream to her entire body.
Why would we need to do this? Julia has been growing a certain bacteria in her lungs, pseudomonas, that is known to cause damage and lower lung function. She has also slowly but surely acquired a cough, appearing during evening treatment sessions, that was never present before pseudomonas took up residence in her lungs. The hope is that with strong IV antibiotics, this bug could be cleared from her lungs, or at least get a good arse kicking and keep it in check for a while.
We won’t be making any decisions yet…waiting for a little more information and some test results. This is something that comes along with this horrible disease. Julia looks perfect on the outside, but every day, every minute there’s a battle going on inside her little body. It’s astonishing what this three year old has to do each day just to be able to breathe…something we all take for granted.
I’m a bit of a mess right now as it takes me an adjustment period when we get new things added onto the dinner plate of Julia’s care. I hate this. I hate CF. We’re just gearing up to fight the good fight and give pseudomonas a beating like no other.
Austin learning monkey skills
Olivia on the rope
Austin can now make it half way up the rope.
The foam block landing pits are a big hit with all the kids.
Hope you all have a fun 4th!
Sweet sisters and their BBQ attire
Ready for church on the 4th of July
4th of July hike
Such a great lookin' bunch!
Just had to put this one in because she's so sweet.
Me, wishing I was the one in the backpack asleep...
This weekend was a great weekend for our family. We couldn’t have asked for better weather as it was much cooler the past two days, even a bit chilly in the evenings. We started the weekend off right by cleaning the house and getting ready for a Saturday evening BBQ. The girls helped me make a flag cake for the occasion and they really enjoyed mixing cake mix after cake mix and putting the fruit touches on the top. The hardest part was making sure Julia didn’t fall into the cake smashing it in it’s entirety.
We had a very tasty meal. The menu included carne asada, fresh tortillas, corn on the cob, rice, beans, seven layer dip, watermelon, fresh cherries, cake, ice cream, and all the fix-in’s you could imagine for some tasty carne asada burritos. I guess having a Mexican-style meal may seem strange for the 4th of July, but I couldn’t resist. I had finally stumbled upon a grocery store that sold carne asada, and I had to have it! I’ve survived six years in Utah, not knowing where to purchase ready-to-grill carne asada and I found it and had to have it. The food was great and I was very pleased with the meal. I even introduced the whole mayo and Parmesan cheese combination for the corn on the cob and some thought it tasted fantastic. If you haven’t tried it, you’re missing out.
Saturday evening we lit fireworks at a near by high school parking lot. Why there? We found out that you can’t light fireworks east of Wasatch Boulevard. We are just one street east, so we are in a no fireworks zone…bummer! We put the fireworks and the kids into the motor home, parked, got out the lawn chairs and had our little family fireworks show.
We headed home around 10 so we could see what big fireworks shows could be viewed from our balcony. We soon found out that from our front porch, you could see multiple fireworks shows. Our best view was of the Sugar House fireworks show. It was quite a treat to get to see the show without fighting the crowds. We could even see Magna’s fireworks show going on way over on the west side. This was the first 4th in this house and we look forward to many more to come.
Since the celebration of the 4th happened on the 3rd, today was just another Sunday. We went to church and then did a short hike with the kids and dog. The kids spotted some butterflies and a lizard and enjoyed picking wild flowers. It was a great way to end the weekend.
I guess I feel the need to reflect a bit today. I feel very grateful to live is such a wonderful country with many opportunities and freedoms. As a woman, I take for granted the ability to wear a pretty little dress to church and show off my Body Pump calves. We don’t think that wearing a tank top is such a privilege, but we don’t live in a country where the only part of a woman that can be exposed is her eyes. The fact that my daughters can go to school and get an education, a higher education, and beyond is a true privilege, a blessing.
We have so much living here in America. I recently finished reading The Power of Two. It is a story about twins who were born with cystic fibrosis and both eventually survived double lung transplants. Joey and I got to hear them speak at the hospital a couple of months ago. These women are half Japanese and they have traveled to Japan many times. What I found simply astonishing is that in Japan, people with cystic fibrosis are lucky to make it past childhood. Japan does not approve many of the medicines that Julia has the privilege of using. My knowledge of the world is so very limited. I do know that we are all so blessed to live here, in this country. We have so much and take so many things for granted.
To switch gears for a minute…it has been quite the roller coaster in the CF community these past two weeks. One beautiful, 28-year-old cyster got her new lungs! So very exciting for Piper. How blessed she is and how excited I am for her to have this new chance at life. The hardest news for me was that of Conner Jones. He is spreading his angel wings and has flown home. The reason this was such difficult news for me is because Conner was Austin’s age and because Conner had cystic fibrosis like Julia. Too close to home for me. I so appreciate his mother Sarah for sharing her story with the CF community and with the world. She has put into words the very intimate details of Conner’s last days, right down to his last meal. I got to see into the world of a very courageous CF parent. Sarah has set such an example for me.
I guess since our family lives with CF everyday there are many unanswered questions that I’ve had. These past two weeks, through Sarah’s blog, I’ve had my questions answered. She painted a very vivid picture of what it was like to be a parent of a child with CF who doesn’t make it past childhood. I think I’m not the only CF parent who wonders if his or her child will ever make it past childhood…wonders what it would be like if the end came sooner than we’d like. The fact is that 50% of people living with CF should expect to live to age 37, but what about the other 50%. The fact is they won’t. I wonder every day what 50% my Julia will be in. I don’t think I’m alone with these thoughts as I’m guessing probably every CF parent wonders the same.
Live every day like it’s your last. Savor the time you have with your families and your children. Our time here is very short, CF or not. Live, love, breathe…
I am a mother to three wonderful children. I submerged myself into motherhood very carefully–one foot at a time. We made sure we had an income sufficient to support a family and medical insurance to go with that. Austin came first, big, healthy…everything perfect. As I think is the case with all firsts, Austin was a challenge for me. Olivia was second. Olivia was a bit harder than Austin, always fussy and constipated. But, she was healthy, strong, and always growing.
With Julia, things were very different. There were red flags from the ultrasound. I had two ultrasounds, both indicating that she had an echogenic bowel. We were told that there was a one in several thousand chance that she could have Down’s Syndrome, or a one in several thousand chance that she could have cystic fibrosis. Naive as I was, I immediately ruled out cystic fibrosis, as we had no family history. I believed if she had anything that she would have Downs and if she had anything at all there was nothing we could do about it at that point anyway.
I did go home and do a little bit of research on cystic fibrosis because I had no idea what it was. I learned that it affected the lungs and digestive system. In the back of my mind I thought that if this new baby developed a cough that wouldn’t go away, then I would get concerned.
Julia came weighing in at 9 pounds 4 ounces. There was meconium in the amniotic fluid during delivery, so as soon as Julia was delivered, she was whisked out the door to have her airways cleared so none would be aspirated. Other than the meconium, which isn’t all that uncommon, things seemed very normal. Julia slept four hours straight that first night in the hospital. I thought finally–I got an easy baby! She breast fed perfectly and pooped a lot, which I thought was so great because my Olivia never pooped.
Julia seemed to be just an average new born. She did get a little cough after a few weeks, but quickly recovered. She was slow on her weight gain, but not dropping off the charts by any means. When I visited my parents when Julia was almost four months, my grandmother made a comment that Julia was fussy, but all my kids were that way, so it didn’t faze me.
It wasn’t until Julia’s four month check up that it finally hit me that something was very wrong. Julia weighed in on that check up at 10 pounds 10 ounces. She was now in the second percentile. To compare…Austin was well in the 18 pound range at four months and Olivia was well into the 16 pound range at that age, so to have Julia still lingering in the 10 pound range was very wrong. After Julia was weighed in by the nurse, we waited for the doctor to come in. As I sat there, I thought of everything I could tell him that seemed strange to me–salty lips when we kissed her, pooping all the time, still nursing every two hours like a newborn would, spitting up excessively, crying all the time, etc. I filled him in on my ultrasound and gave him all I could think of in terms of Julia’s behaviors. I mentioned cystic fibrosis in that meeting. I could just see the look on his face and I knew that he already suspected it.
He sent us up to Primary Children’s Hospital immediately. We left the doctor’s office and headed straight up there. I called my husband and told him to meet us up there and take Austin and Olivia to lunch while Julia was sweat tested. They sweated her and took blood for labs. I knew she had CF and was just waiting for the call that followed that evening with the final confirmation. It was so hard to wait. I got on line and read all I could about cystic fibrosis. After all my reading, I truly felt that cystic fibrosis was an awful thing to have…something you wouldn’t wish on your worst enemy.
I got the call around five that evening and the sweat test was indeed positive for cystic fibrosis. No surprise, but it didn’t make hearing it any easier. We were to check into Primary Children’s the next morning. I cried a lot that night. It should have been just like any other night, but I thought for some reason that Julia might not make it through the night having been starved for so long. I felt her feedings that night were completely pointless as now I was aware that it was all going in one end and just passing out the other side.
We stayed for two days at the hospital with Julia. It was like a two day training seminar on cystic fibrosis and how to care for Julia. The most important thing was to get her on enzymes and to get her gaining weight. The enzymes were very challenging for Julia. She was only four months and hadn’t eating anything solid her entire life, so she was gagging down applesauce with enzyme granules on top. I was afraid she would inhale it into her lungs and we’d end up with a bigger problem. It took a lot of practice.
Another issue was that I was breast feeding Julia and she needed more calories. Big dilemma! I wanted to breast feed, but I knew she needed more. Being the person I am, I wanted her to have the best of both worlds, so I was determined to get her on a bottle, pump, and add formula to breast milk using the recipe they gave us at the hospital. That would have to wait until we got home.
My husband and I were very overwhelmed at the hospital. When we thought there couldn’t be any more to this disease, people would come in and give us more, and more, and more. For example, when we were getting trained on chest physical therapy, in my mind I would think, “OK, I can do this,” thinking it would only be when she was actually sick and coughing. Then my bubble would burst when I was told, “No, it would be every day…for the rest of her life…oh and not only once a day, but twice a day, or more than that if she’s sick.” What! Are you kidding me! My bubble was burst so many times like that and I felt like I was drowning. That’s looking at it from my selfish side…not to mention how a four month old was going to handle all of this.
They didn’t start us on the lung treatments right away. I was so glad for that. I needed some time just to get Julia’s diet under control. We got home and felt completely depressed and helpless. I cried a lot as I was going through some kind of mourning process. I went to work on pumping and getting her to take a bottle. We experimented with every nipple we could get our hands on. There was a lot of screaming and resistance, but we finally got her to take the Dr. Brown’s bottle. The nipple was much softer and more pliable than many of the other brands. What I didn’t like about that bottle is all of the extra parts I had to wash with the whole air vent system.
So, we got Julia taking her Prevacid and Creon 5 enzymes as well as pumping by day and power packing the breast milk. I didn’t pump at night. At night I just fed her from the breast. I figured I needed the rest and felt that what I was doing during the day was sufficient. Julia was having her cake and eating it too. I was exhausted, but felt good that she was getting the best I could give her. I wouldn’t recommend this for all new mothers. It was very difficult and it would have been much easier just to do straight formula in the high calorie recipe.
A year after Julia was born, the state of Utah passed a law that included cystic fibrosis in the newborn screening tests. This a wonderful thing, allowing babies to be diagnosed at a very early age. For me, as hard as it was, I am somewhat glad that I got to see, first hand, the effects of this disease. I’m not a person that relies on faith to a great extent…I’m more of a ”
show me the money” type of person. I think it would be hard as a new parent to have a health care professional tell you that your newborn has CF and here is all that goes with that. It would be hard to make a newborn gag down enzymes if you never got to see the harmful effects of enzyme free living in a person with CF. For this I am grateful. CF is very devastating if left untreated. If your newborn is positive for CF, trust that the medications and treatments are essential for optimal health. I know because I’ve seen what it does and it’s awful.
This is how our family began our journey with our little CF angel. Having a child with cystic fibrosis is a very bumpy road. As if being a mother isn’t hard enough…you throw CF into the mix and you literally have to become superwoman over night. This journey has transformed me in ways I could have never imagined. I am a completely different person because of Julia. She is such a strong spirit and seems to have come programmed to be tough, durable, meek, submissive, patient, and so much more. She teaches me every day that it’s not about me…as much as I would like everything to be about me…it just can’t be. I have to force myself to be completely selfless when it comes to this disease and that’s a very hard thing to do.
Having said that, I found it essential that I take a little me time. I found myself on a downward spiral and I knew that if I didn’t step away from Julia for a bit I would find myself on some medication very quickly. I began working a few hours a week with my husband’s business to step away and get my mind in a different place. I also began hitting the gym, taking spinning classes and again, getting my mind in a different place. I truly felt that if I didn’t do this, I was going to go crazy, literally. These outlets saved my sanity for sure.
This is just the beginning and I hope the end never comes in my lifetime. I love my life, my kids, my husband, and all of the crazy twists and turns that come with it. This is what makes me who I am.